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Volumn 8, Issue 3, 2009, Pages 137-154

Animal models of human cerebellar ataxias: A cornerstone for the therapies of the twenty-first century

Author keywords

Animal models; Ataxias; Cerebellum; Pathogenesis; Therapies

Indexed keywords

ATAXIA; CEREBELLAR ATAXIA; HEREDITARY ATAXIA; MOUSE STRAIN; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; REVIEW; SCREENING; SPINOCEREBELLAR DEGENERATION; X CHROMOSOME LINKED DISORDER; X LINKED ATAXIA;

EID: 69549116455     PISSN: 14734222     EISSN: 14734230     Source Type: Journal    
DOI: 10.1007/s12311-009-0127-3     Document Type: Review
Times cited : (34)

References (247)
  • 1
    • 58149097467 scopus 로고    scopus 로고
    • The cerebellum, cerebellar disorders, and cerebellar research-two centuries of discoveries
    • M Manto 2008 The cerebellum, cerebellar disorders, and cerebellar research-two centuries of discoveries Cerebellum 7 4 505 516
    • (2008) Cerebellum , vol.7 , Issue.4 , pp. 505-516
    • Manto, M.1
  • 2
    • 65849288724 scopus 로고    scopus 로고
    • Spinocerebellar ataxias caused by polyglutamine expansions: A review of therapeutic strategies
    • BR Underwood DC Rubinsztein 2008 Spinocerebellar ataxias caused by polyglutamine expansions: a review of therapeutic strategies Cerebellum 7 2 215 221
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 215-221
    • Underwood, B.R.1    Rubinsztein, D.C.2
  • 4
    • 65849230539 scopus 로고    scopus 로고
    • The clinical diagnosis of autosomal dominant spinocerebellar ataxias
    • T Klockgether 2008 The clinical diagnosis of autosomal dominant spinocerebellar ataxias Cerebellum 7 2 101 105
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 101-105
    • Klockgether, T.1
  • 5
    • 53849092039 scopus 로고    scopus 로고
    • The cerebellum is involved in reward-based reversal learning
    • P Thoma C Bellebaum B Koch M Schwarz I Daum 2008 The cerebellum is involved in reward-based reversal learning Cerebellum 7 3 433 443
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 433-443
    • Thoma, P.1    Bellebaum, C.2    Koch, B.3    Schwarz, M.4    Daum, I.5
  • 7
    • 44949163101 scopus 로고    scopus 로고
    • Cognitive sequencing impairment in patients with focal or atrophic cerebellar damage
    • MG Leggio AM Tedesco FR Chiricozzi S Clausi A Orsini M Molinari 2008 Cognitive sequencing impairment in patients with focal or atrophic cerebellar damage Brain 131 Pt 5 1332 1343
    • (2008) Brain , vol.131 , Issue.PART 5 , pp. 1332-1343
    • Leggio, M.G.1    Tedesco, A.M.2    Chiricozzi, F.R.3    Clausi, S.4    Orsini, A.5    Molinari, M.6
  • 8
    • 34548406075 scopus 로고    scopus 로고
    • Cerebellar contributions to cognitive functions: A progress report after two decades of research
    • D Timmann I Daum 2007 Cerebellar contributions to cognitive functions: a progress report after two decades of research Cerebellum 6 3 159 162
    • (2007) Cerebellum , vol.6 , Issue.3 , pp. 159-162
    • Timmann, D.1    Daum, I.2
  • 9
    • 9444224946 scopus 로고    scopus 로고
    • Small de novo duplication in the repeat region of the TATA-box-binding protein gene manifest with a phenotype similar to variant Creutzfeldt-Jakob disease
    • A Shatunov EA Fridman FI Pagan J Leib A Singleton M Hallett LG Goldfarb 2004 Small de novo duplication in the repeat region of the TATA-box-binding protein gene manifest with a phenotype similar to variant Creutzfeldt-Jakob disease Clin Genet 66 6 496 501
    • (2004) Clin Genet , vol.66 , Issue.6 , pp. 496-501
    • Shatunov, A.1    Fridman, E.A.2    Pagan, F.I.3    Leib, J.4    Singleton, A.5    Hallett, M.6    Goldfarb, L.G.7
  • 10
    • 34548390222 scopus 로고    scopus 로고
    • Cognition in hereditary ataxia
    • K Bürk 2007 Cognition in hereditary ataxia Cerebellum 6 3 280 286
    • (2007) Cerebellum , vol.6 , Issue.3 , pp. 280-286
    • Bürk, K.1
  • 11
    • 54249085676 scopus 로고    scopus 로고
    • The highly heterogeneous spinocerebellar ataxias: From genes to targets for therapeutic intervention
    • A Matilla-Dueñas 2008 The highly heterogeneous spinocerebellar ataxias: from genes to targets for therapeutic intervention Cerebellum 7 2 97 100
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 97-100
    • Matilla-Dueñas, A.1
  • 12
    • 54949149113 scopus 로고    scopus 로고
    • Hereditary episodic ataxias
    • JC Jen 2008 Hereditary episodic ataxias Ann N Y Acad Sci 1142 250 253
    • (2008) Ann N y Acad Sci , vol.1142 , pp. 250-253
    • Jen, J.C.1
  • 13
    • 65849214710 scopus 로고    scopus 로고
    • Clinical, genetic, molecular, and pathophysiological insights into spinocerebellar ataxia type 1
    • A Matilla-Dueñas R Goold P Giunti 2008 Clinical, genetic, molecular, and pathophysiological insights into spinocerebellar ataxia type 1 Cerebellum 7 2 106 114
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 106-114
    • Matilla-Dueñas, A.1    Goold, R.2    Giunti, P.3
  • 14
    • 33745088678 scopus 로고    scopus 로고
    • Molecular pathogenesis of spinocerebellar ataxias
    • AM Dueñas R Goold P Giunti 2006 Molecular pathogenesis of spinocerebellar ataxias Brain 129 Pt 6 1357 1370
    • (2006) Brain , vol.129 , Issue.PART 6 , pp. 1357-1370
    • Dueñas, A.M.1    Goold, R.2    Giunti, P.3
  • 17
    • 1442280669 scopus 로고    scopus 로고
    • Dynamic mutation and human disorders: The spinocerebellar ataxias
    • MA Costa Lima MM Pimentel 2004 Dynamic mutation and human disorders: the spinocerebellar ataxias Int J Mol Med 13 2 299 302
    • (2004) Int J Mol Med , vol.13 , Issue.2 , pp. 299-302
    • Costa Lima, M.A.1    Pimentel, M.M.2
  • 18
    • 61449238441 scopus 로고    scopus 로고
    • Bidirectional expression of the SCA8 expansion mutation: One mutation, two genes
    • Y Ikeda RS Daughters LP Ranum 2008 Bidirectional expression of the SCA8 expansion mutation: one mutation, two genes Cerebellum 7 2 150 158
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 150-158
    • Ikeda, Y.1    Daughters, R.S.2    Ranum, L.P.3
  • 21
    • 33747651583 scopus 로고    scopus 로고
    • The role of ataxin 10 in the pathogenesis of spinocerebellar ataxia type 10
    • DOI 10.1212/01.wnl.0000231140.26253.eb, PII 0000611420060822000018
    • M Wakamiya T Matsuura Y Liu GC Schuster R Gao W Xu PS Sarkar X Lin T Ashizawa 2006 The role of ataxin 10 in the pathogenesis of spinocerebellar ataxia type 10 Neurology 67 4 607 613 (Pubitemid 44273606)
    • (2006) Neurology , vol.67 , Issue.4 , pp. 607-613
    • Wakamiya, M.1    Matsuura, T.2    Liu, Y.3    Schuster, G.C.4    Gao, R.5    Xu, W.6    Sarkar, P.S.7    Lin, X.8    Ashizawa, T.9
  • 22
    • 33845524420 scopus 로고    scopus 로고
    • Diagnosis and management of early- and late-onset cerebellar ataxia
    • E Brusse JA Maat-Kievit JC van Swieten 2007 Diagnosis and management of early- and late-onset cerebellar ataxia Clin Genet 71 1 12 24
    • (2007) Clin Genet , vol.71 , Issue.1 , pp. 12-24
    • Brusse, E.1    Maat-Kievit, J.A.2    Van Swieten, J.C.3
  • 23
    • 65849376966 scopus 로고    scopus 로고
    • Sporadic ataxias in Japan-a population-based epidemiological study
    • Study Group on Ataxic Diseases
    • S Tsuji O Onodera J Goto M Nishizawa Study Group on Ataxic Diseases 2008 Sporadic ataxias in Japan-a population-based epidemiological study Cerebellum 7 2 189 197
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 189-197
    • Tsuji, S.1    Onodera, O.2    Goto, J.3    Nishizawa, M.4
  • 25
    • 53849120554 scopus 로고    scopus 로고
    • Mechanisms of ethanol-induced degeneration in the developing, mature, and aging cerebellum
    • P Jaatinen J Rintala 2008 Mechanisms of ethanol-induced degeneration in the developing, mature, and aging cerebellum Cerebellum 7 3 332 347
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 332-347
    • Jaatinen, P.1    Rintala, J.2
  • 29
    • 18444386197 scopus 로고    scopus 로고
    • A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration
    • K Watase EJ Weeber B Xu B Antalffy L Yuva-Paylor K Hashimoto, et al. 2002 A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration Neuron 34 905 919
    • (2002) Neuron , vol.34 , pp. 905-919
    • Watase, K.1    Weeber, E.J.2    Xu, B.3    Antalffy, B.4    Yuva-Paylor, L.5    Hashimoto, K.6
  • 30
    • 5444248681 scopus 로고    scopus 로고
    • Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice
    • T Zu LA Duvick MD Kaytor MS Berlinger HY Zoghbi HB Clark, et al. 2004 Recovery from polyglutamine-induced neurodegeneration in conditional SCA1 transgenic mice J Neurosci 24 8853 8861
    • (2004) J Neurosci , vol.24 , pp. 8853-8861
    • Zu, T.1    Duvick, L.A.2    Kaytor, M.D.3    Berlinger, M.S.4    Zoghbi, H.Y.5    Clark, H.B.6
  • 31
    • 0031838352 scopus 로고    scopus 로고
    • Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
    • CJ Cummings MA Mancini B Antalffy DB DeFranco HT Orr HY Zoghbi 1998 Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1 Nat Genet 19 148 154
    • (1998) Nat Genet , vol.19 , pp. 148-154
    • Cummings, C.J.1    Mancini, M.A.2    Antalffy, B.3    Defranco, D.B.4    Orr, H.T.5    Zoghbi, H.Y.6
  • 32
    • 0030864463 scopus 로고    scopus 로고
    • Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations
    • HB Clark EN Burright WS Yunis S Larson C Wilcox B Hartman, et al. 1997 Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations J Neurosci 17 7385 7395
    • (1997) J Neurosci , vol.17 , pp. 7385-7395
    • Clark, H.B.1    Burright, E.N.2    Yunis, W.S.3    Larson, S.4    Wilcox, C.5    Hartman, B.6
  • 33
    • 0029163222 scopus 로고
    • SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat
    • EN Burright HB Clark A Servadio T Matilla RM Feddersen WS Yunis, et al. 1995 SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat Cell 82 937 948
    • (1995) Cell , vol.82 , pp. 937-948
    • Burright, E.N.1    Clark, H.B.2    Servadio, A.3    Matilla, T.4    Feddersen, R.M.5    Yunis, W.S.6
  • 34
    • 0033811788 scopus 로고    scopus 로고
    • Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human
    • DP Huynh K Figueroa N Hoang SM Pulst 2000 Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human Nat Genet 26 44 50
    • (2000) Nat Genet , vol.26 , pp. 44-50
    • Huynh, D.P.1    Figueroa, K.2    Hoang, N.3    Pulst, S.M.4
  • 35
    • 0036566229 scopus 로고    scopus 로고
    • YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit
    • CK Cemal CJ Carroll L Lawrence MB Lowrie P Ruddle S Al-Mahdawi, et al. 2002 YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit Hum Mol Genet 11 1075 1094
    • (2002) Hum Mol Genet , vol.11 , pp. 1075-1094
    • Cemal, C.K.1    Carroll, C.J.2    Lawrence, L.3    Lowrie, M.B.4    Ruddle, P.5    Al-Mahdawi, S.6
  • 36
    • 20844462057 scopus 로고    scopus 로고
    • A mutant ataxin-3 putative-cleavage fragment in brain of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration
    • D Goti SM Katzen J Mez N Kurtis J Kiluk L Ben-Haïem, et al. 2004 A mutant ataxin-3 putative-cleavage fragment in brain of Machado-Joseph disease patients and transgenic mice is cytotoxic above a critical concentration J Neurosci 24 10266 10279
    • (2004) J Neurosci , vol.24 , pp. 10266-10279
    • Goti, D.1    Katzen, S.M.2    Mez, J.3    Kurtis, N.4    Kiluk, J.5    Ben-Haïem, L.6
  • 40
    • 0037421691 scopus 로고    scopus 로고
    • SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity
    • SY Yoo ME Pennesi EJ Weeber B Xu R Atkinson S Chen, et al. 2003 SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity Neuron 37 383 401
    • (2003) Neuron , vol.37 , pp. 383-401
    • Yoo, S.Y.1    Pennesi, M.E.2    Weeber, E.J.3    Xu, B.4    Atkinson, R.5    Chen, S.6
  • 41
    • 0034641891 scopus 로고    scopus 로고
    • Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice
    • G Yvert KS Lindenberg S Picaud GB Landwehrmeyer JA Sahel JL Mandel 2000 Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice Hum Mol Genet 9 2491 2506
    • (2000) Hum Mol Genet , vol.9 , pp. 2491-2506
    • Yvert, G.1    Lindenberg, K.S.2    Picaud, S.3    Landwehrmeyer, G.B.4    Sahel, J.A.5    Mandel, J.L.6
  • 42
    • 0037096365 scopus 로고    scopus 로고
    • Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous Purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice
    • GA Garden RT Libby YH Fu Y Kinoshita J Huang DE Possin, et al. 2002 Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous Purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice J Neurosci 22 4897 4905
    • (2002) J Neurosci , vol.22 , pp. 4897-4905
    • Garden, G.A.1    Libby, R.T.2    Fu, Y.H.3    Kinoshita, Y.4    Huang, J.5    Possin, D.E.6
  • 44
    • 33749170168 scopus 로고    scopus 로고
    • Targeted deletion of a single Sca8 ataxia locus allele in mice causes abnormal gait, progressive loss of motor coordination, and Purkinje cell dendritic deficits
    • Y He T Zu KA Benzow HT Orr HB Clark MD Koob 2006 Targeted deletion of a single Sca8 ataxia locus allele in mice causes abnormal gait, progressive loss of motor coordination, and Purkinje cell dendritic deficits J Neurosci 26 39 9975 9982
    • (2006) J Neurosci , vol.26 , Issue.39 , pp. 9975-9982
    • He, Y.1    Zu, T.2    Benzow, K.A.3    Orr, H.T.4    Clark, H.B.5    Koob, M.D.6
  • 45
    • 0346656870 scopus 로고    scopus 로고
    • Specific isoforms of protein kinase C are essential for prevention of folate-resistant neural tube defects byinositol
    • P Cogram A Hynes LP Dunlevy ND Greene AJ Copp 2004 Specific isoforms of protein kinase C are essential for prevention of folate-resistant neural tube defects byinositol Hum Mol Genet 13 7 14
    • (2004) Hum Mol Genet , vol.13 , pp. 7-14
    • Cogram, P.1    Hynes, A.2    Dunlevy, L.P.3    Greene, N.D.4    Copp, A.J.5
  • 47
    • 0034704187 scopus 로고    scopus 로고
    • Distinct roles for TBP and TBP-like factor in early embryonic gene transcription in Xenopus
    • GJC Veenstra DL Weeks AP Wolffe 2000 Distinct roles for TBP and TBP-like factor in early embryonic gene transcription in Xenopus Science 290 2312 2314
    • (2000) Science , vol.290 , pp. 2312-2314
    • Veenstra, G.J.C.1    Weeks, D.L.2    Wolffe, A.P.3
  • 48
    • 36448930958 scopus 로고    scopus 로고
    • Polyglutamine domain modulates the TBP-TFIIB interaction: Implications for its normal function and neurodegeneration
    • MJ Friedman AG Shah ZH Fang EG Ward ST Warren S Li XJ Li 2007 Polyglutamine domain modulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration Nat Neurosci 10 12 1519 1528
    • (2007) Nat Neurosci , vol.10 , Issue.12 , pp. 1519-1528
    • Friedman, M.J.1    Shah, A.G.2    Fang, Z.H.3    Ward, E.G.4    Warren, S.T.5    Li, S.6    Li, X.J.7
  • 49
    • 0037067646 scopus 로고    scopus 로고
    • Removing the vertebrate-specific TBP N terminus disrupts placental beta-2M-dependent interactions with the maternal immune system
    • NK Hobbs AA Bondareva S Barnett MR Capecchi EE Schmidt 2002 Removing the vertebrate-specific TBP N terminus disrupts placental beta-2M-dependent interactions with the maternal immune system Cell 110 43 54
    • (2002) Cell , vol.110 , pp. 43-54
    • Hobbs, N.K.1    Bondareva, A.A.2    Barnett, S.3    Capecchi, M.R.4    Schmidt, E.E.5
  • 50
    • 0036829688 scopus 로고    scopus 로고
    • RNA polymerase II transcription in murine cells lacking the TATA binding protein
    • I Martianov S Viville I Davidson 2002 RNA polymerase II transcription in murine cells lacking the TATA binding protein Science 298 1036 1039
    • (2002) Science , vol.298 , pp. 1036-1039
    • Martianov, I.1    Viville, S.2    Davidson, I.3
  • 54
    • 0035138072 scopus 로고    scopus 로고
    • Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits
    • H Puccio D Simon M Cossée P Criqui-Filipe F Tiziano J Melki C Hindelang R Matyas P Rustin M Koenig 2001 Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits Nat Genet 27 181 186
    • (2001) Nat Genet , vol.27 , pp. 181-186
    • Puccio, H.1    Simon, D.2    Cossée, M.3    Criqui-Filipe, P.4    Tiziano, F.5    Melki, J.6    Hindelang, C.7    Matyas, R.8    Rustin, P.9    Koenig, M.10
  • 56
    • 1442324707 scopus 로고    scopus 로고
    • Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia
    • D Simon H Seznec A Gansmuller N Carelle P Weber D Metzger P Rustin M Koenig H Puccio 2004 Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia J Neurosci 24 1987 1995
    • (2004) J Neurosci , vol.24 , pp. 1987-1995
    • Simon, D.1    Seznec, H.2    Gansmuller, A.3    Carelle, N.4    Weber, P.5    Metzger, D.6    Rustin, P.7    Koenig, M.8    Puccio, H.9
  • 58
    • 30744458144 scopus 로고    scopus 로고
    • Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant
    • RP Vazquez-Manrique P Gonzalez-Cabo S Ros H Aziz HA Baylis F Palau 2006 Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant FASEB J 20 172 174
    • (2006) FASEB J , vol.20 , pp. 172-174
    • Vazquez-Manrique, R.P.1    Gonzalez-Cabo, P.2    Ros, S.3    Aziz, H.4    Baylis, H.A.5    Palau, F.6
  • 59
    • 27944495710 scopus 로고    scopus 로고
    • RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila
    • PR Anderson K Kirby AJ Hilliker JP Phillips 2005 RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila Hum Mol Genet 14 3397 3405
    • (2005) Hum Mol Genet , vol.14 , pp. 3397-3405
    • Anderson, P.R.1    Kirby, K.2    Hilliker, A.J.3    Phillips, J.P.4
  • 60
    • 0030063759 scopus 로고    scopus 로고
    • The COQ7 gene encodes a protein in Saccharomyces cerevisiae necessary for ubiquinone biosynthesis
    • BN Marbois CF Clarke 1996 The COQ7 gene encodes a protein in Saccharomyces cerevisiae necessary for ubiquinone biosynthesis J Biol Chem 271 2995 3004
    • (1996) J Biol Chem , vol.271 , pp. 2995-3004
    • Marbois, B.N.1    Clarke, C.F.2
  • 61
    • 0035895199 scopus 로고    scopus 로고
    • A dietary source of coenzyme Q is essential for growth of long-lived Caenorhabditis elegans clk-1 mutants
    • T Jonassen PL Larsen CF Clarke 2001 A dietary source of coenzyme Q is essential for growth of long-lived Caenorhabditis elegans clk-1 mutants Proc Natl Acad Sci U S A 98 421 426
    • (2001) Proc Natl Acad Sci U S A , vol.98 , pp. 421-426
    • Jonassen, T.1    Larsen, P.L.2    Clarke, C.F.3
  • 62
    • 0035976759 scopus 로고    scopus 로고
    • Mouse homologue of coq7/ clk-1, longevity gene in Caenorhabditis elegans, is essential for coenzyme Q synthesis, maintenance of mitochondrial integrity, and neurogenesis
    • D Nakai S Yuasa M Takahashi, et al. 2001 Mouse homologue of coq7/ clk-1, longevity gene in Caenorhabditis elegans, is essential for coenzyme Q synthesis, maintenance of mitochondrial integrity, and neurogenesis Biochem Biophys Res Commun 289 463 471
    • (2001) Biochem Biophys Res Commun , vol.289 , pp. 463-471
    • Nakai, D.1    Yuasa, S.2    Takahashi, M.3
  • 63
    • 0035824510 scopus 로고    scopus 로고
    • Ubiquinone is necessary for mouse embryonic development but is not essential for mitochondrial respiration
    • F Levavasseur H Miyadera J Sirois, et al. 2001 Ubiquinone is necessary for mouse embryonic development but is not essential for mitochondrial respiration J Biol Chem 276 46160 46164
    • (2001) J Biol Chem , vol.276 , pp. 46160-46164
    • Levavasseur, F.1    Miyadera, H.2    Sirois, J.3
  • 67
    • 25144520251 scopus 로고    scopus 로고
    • Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP
    • L Zhao C Longo-Guess BS Harris J-W Lee SL Ackerman 2005 Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP Nature Genet 37 974 979
    • (2005) Nature Genet , vol.37 , pp. 974-979
    • Zhao, L.1    Longo-Guess, C.2    Harris, B.S.3    Lee, J.-W.4    Ackerman, S.L.5
  • 75
    • 0037089319 scopus 로고    scopus 로고
    • Paradoxical enhancement of hepatic metabolism of 7- ketocholesterol in sterol 27- hydroxylase-deficient mice
    • MA Lyons N Maeda AJ Brown 2002 Paradoxical enhancement of hepatic metabolism of 7- ketocholesterol in sterol 27- hydroxylase-deficient mice Biochim Biophys Acta 1581 3 119 126
    • (2002) Biochim Biophys Acta , vol.1581 , Issue.3 , pp. 119-126
    • Lyons, M.A.1    Maeda, N.2    Brown, A.J.3
  • 76
    • 0014472414 scopus 로고
    • Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide containing hydroxy fatty acid
    • P Morell NS Radin 1969 Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide containing hydroxy fatty acid Biochemistry 8 506 512
    • (1969) Biochemistry , vol.8 , pp. 506-512
    • Morell, P.1    Radin, N.S.2
  • 77
    • 0034885992 scopus 로고    scopus 로고
    • Lysosomal sulfoglycolipid storage in the kidneys of mice deficient for arylsulfatase A (ASA) and of double-knockout mice deficient for ASA and galactosylceramide synthase
    • R Lüllmann-Rauch U Matzner S Franken D Hartmann V Gieselmann 2001 Lysosomal sulfoglycolipid storage in the kidneys of mice deficient for arylsulfatase A (ASA) and of double-knockout mice deficient for ASA and galactosylceramide synthase Histochem Cell Biol 116 161 169
    • (2001) Histochem Cell Biol , vol.116 , pp. 161-169
    • Lüllmann-Rauch, R.1    Matzner, U.2    Franken, S.3    Hartmann, D.4    Gieselmann, V.5
  • 79
    • 0019977775 scopus 로고
    • Lysosome lipid storage disorder in NCTR-BALB/c mice. I. Description of the disease and genetics
    • MD Morris C Bhuvaneswaran H Shio S Fowler 1982 Lysosome lipid storage in NCTR-BALB/c mice. I. Description of the disease and genetics Am J Pathol 108 140 149 (Pubitemid 12051674)
    • (1982) American Journal of Pathology , vol.108 , Issue.2 , pp. 140-149
    • Morris, M.D.1    Bhuvaneswaran, C.2    Shio, H.3    Fowler, S.4
  • 80
    • 0034192462 scopus 로고    scopus 로고
    • A model for Niemann-Pick type C disease in the nematode Caenorhabditis elegans
    • M Sym M Basson C Johnson 2000 A model for Niemann-Pick type C disease in the nematode Caenorhabditis elegans Curr Biol 10 9 527 530
    • (2000) Curr Biol , vol.10 , Issue.9 , pp. 527-530
    • Sym, M.1    Basson, M.2    Johnson, C.3
  • 81
    • 0015329201 scopus 로고
    • GM gangliosidosis in a cat
    • WF Blakemore 1972 GM gangliosidosis in a cat J Comp Pathol 82 179
    • (1972) J Comp Pathol , vol.82 , pp. 179
    • Blakemore, W.F.1
  • 82
    • 0043101323 scopus 로고
    • Gm1-gangliosidosis of Friesian calves. A review
    • WJC Donnelly BJ Sheahan 1981 Gm1-gangliosidosis of Friesian calves. A review Irish Vet J 35 45 55
    • (1981) Irish Vet J , vol.35 , pp. 45-55
    • Donnelly, W.J.C.1    Sheahan, B.J.2
  • 83
    • 0025089547 scopus 로고
    • Clinico-pathological studies of LEC rats with hereditary hepatitis and hepatoma in the acute phase of hepatitis
    • N Kasai T Osanai I Miyoshi E Kamimura MC Yoshida K Dempo 1990 Clinico-pathological studies of LEC rats with hereditary hepatitis and hepatoma in the acute phase of hepatitis Lab Anim Sci 40 502 505
    • (1990) Lab Anim Sci , vol.40 , pp. 502-505
    • Kasai, N.1    Osanai, T.2    Miyoshi, I.3    Kamimura, E.4    Yoshida, M.C.5    Dempo, K.6
  • 84
    • 0032875387 scopus 로고    scopus 로고
    • Targeted gene disruption reveals an essential role for ceruloplasmin in cellular iron efflux
    • ZL Harris AP Durley TK Man JD Gitlin 1999 Targeted gene disruption reveals an essential role for ceruloplasmin in cellular iron efflux Proc Natl Acad Sci U S A 96 19 10812 10817
    • (1999) Proc Natl Acad Sci U S A , vol.96 , Issue.19 , pp. 10812-10817
    • Harris, Z.L.1    Durley, A.P.2    Man, T.K.3    Gitlin, J.D.4
  • 86
    • 0032948849 scopus 로고    scopus 로고
    • Atm haploinsufficiency results in increased sensitivity to sublethal doses of ionizing radiation in mice
    • C Barlow MA Eckhaus AA Schaffer A Wynshaw-Boris 1999 Atm haploinsufficiency results in increased sensitivity to sublethal doses of ionizing radiation in mice Nature Genet 21 359 360
    • (1999) Nature Genet , vol.21 , pp. 359-360
    • Barlow, C.1    Eckhaus, M.A.2    Schaffer, A.A.3    Wynshaw-Boris, A.4
  • 88
    • 13844316424 scopus 로고    scopus 로고
    • The fragile X mental retardation protein and group i metabotropic glutamate receptors regulate levels of mRNA granules in brain
    • A Aschrafi BA Cunningham GM Edelman PW Vanderklish 2005 The fragile X mental retardation protein and group I metabotropic glutamate receptors regulate levels of mRNA granules in brain Proc Nat Acad Sci U S A 102 2180 2185
    • (2005) Proc Nat Acad Sci U S A , vol.102 , pp. 2180-2185
    • Aschrafi, A.1    Cunningham, B.A.2    Edelman, G.M.3    Vanderklish, P.W.4
  • 92
    • 0033581027 scopus 로고    scopus 로고
    • Preventing neurodegeneration in the Drosophila mutant bubblegum
    • KT Min S Benzer 1999 Preventing neurodegeneration in the Drosophila mutant bubblegum Science 284 1985 1988
    • (1999) Science , vol.284 , pp. 1985-1988
    • Min, K.T.1    Benzer, S.2
  • 93
    • 0030071685 scopus 로고    scopus 로고
    • The methyl-CpG binding protein MeCP2 is essential for embryonic development in the mouse
    • P Tate W Skarnes A Bird 1996 The methyl-CpG binding protein MeCP2 is essential for embryonic development in the mouse Nature Genet 12 205
    • (1996) Nature Genet , vol.12 , pp. 205
    • Tate, P.1    Skarnes, W.2    Bird, A.3
  • 94
    • 1542344372 scopus 로고    scopus 로고
    • X-chromosome inactivation patterns are unbalanced and affect the phenotypic outcome in a mouse model of Rett syndrome
    • JI Young HY Zoghbi 2004 X-chromosome inactivation patterns are unbalanced and affect the phenotypic outcome in a mouse model of Rett syndrome Am J Hum Genet 74 511 520
    • (2004) Am J Hum Genet , vol.74 , pp. 511-520
    • Young, J.I.1    Zoghbi, H.Y.2
  • 95
    • 33947150414 scopus 로고    scopus 로고
    • Rodent models of tremor
    • H Miwa 2007 Rodent models of tremor Cerebellum 6 1 66 72
    • (2007) Cerebellum , vol.6 , Issue.1 , pp. 66-72
    • Miwa, H.1
  • 97
    • 33751397715 scopus 로고    scopus 로고
    • Injury severity determines Purkinje cell loss and microglial activation in the cerebellum after cortical contusion injury
    • T Igarashi MB Potts LJ Noble-Haeusslein 2007 Injury severity determines Purkinje cell loss and microglial activation in the cerebellum after cortical contusion injury Exp Neurol 203 1 258 268
    • (2007) Exp Neurol , vol.203 , Issue.1 , pp. 258-268
    • Igarashi, T.1    Potts, M.B.2    Noble-Haeusslein, L.J.3
  • 98
    • 67949109759 scopus 로고    scopus 로고
    • Animal models to study thyroid hormone action in cerebellum
    • N Koibuchi 2009 Animal models to study thyroid hormone action in cerebellum Cerebellum 8 2 89 97
    • (2009) Cerebellum , vol.8 , Issue.2 , pp. 89-97
    • Koibuchi, N.1
  • 99
    • 33847709488 scopus 로고    scopus 로고
    • Spontaneous and induced mouse mutations with cerebellar dysfunctions: Behavior and neurochemistry
    • R Lalonde C Strazielle 2007 Spontaneous and induced mouse mutations with cerebellar dysfunctions: behavior and neurochemistry Brain Res 1140 51 74
    • (2007) Brain Res , vol.1140 , pp. 51-74
    • Lalonde, R.1    Strazielle, C.2
  • 100
    • 0030800468 scopus 로고    scopus 로고
    • Neurodegeneration in Lurcher mutant mice caused by mutation in the delta 2 glutamate receptor gene
    • J Zuo PI De Jager KA Takahashi W Jiang DJ Linden N Heintz 1997 Neurodegeneration in Lurcher mutant mice caused by mutation in the delta 2 glutamate receptor gene Nature 388 769 773
    • (1997) Nature , vol.388 , pp. 769-773
    • Zuo, J.1    De Jager, P.I.2    Takahashi, K.A.3    Jiang, W.4    Linden, D.J.5    Heintz, N.6
  • 101
    • 0035958636 scopus 로고    scopus 로고
    • Neurobiological effects of a null mutation depend on genetic context: Comparison between two hotfoot alleles of the delta-2 ionotropic glutamate receptor
    • A Lalouette A Lohof C Sotelo J Guénet J Mariani 2001 Neurobiological effects of a null mutation depend on genetic context: comparison between two hotfoot alleles of the delta-2 ionotropic glutamate receptor Neuroscience 105 443 455
    • (2001) Neuroscience , vol.105 , pp. 443-455
    • Lalouette, A.1    Lohof, A.2    Sotelo, C.3    Guénet, J.4    Mariani, J.5
  • 102
    • 0029035706 scopus 로고
    • The mouse dystonia musculorum gene is a neural isoform of bullous pemphigoid antigen 1
    • A Brown G Bernier M Mathieu J Rossant R Kothary 1995 The mouse dystonia musculorum gene is a neural isoform of bullous pemphigoid antigen 1 Nat Genet 10 301 306
    • (1995) Nat Genet , vol.10 , pp. 301-306
    • Brown, A.1    Bernier, G.2    Mathieu, M.3    Rossant, J.4    Kothary, R.5
  • 103
    • 69549112947 scopus 로고    scopus 로고
    • Pogo: A novel spontaneous ataxic mutant mouse
    • doi: 10.1007/s12311-009-0096-6
    • Lee NS, Jeong YG (2009) Pogo: a novel spontaneous ataxic mutant mouse. Cerebellum. doi: 10.1007/s12311-009-0096-6
    • (2009) Cerebellum
    • Lee, N.S.1    Jeong, Y.G.2
  • 105
    • 0031064169 scopus 로고    scopus 로고
    • Mutations in the Cacnl1a4 calcium channel gene are associated with seizures, cerebellar degeneration, and ataxia in tottering and leaner mutant mice
    • J Doyle X Ren G Lennon L Stubbs 1997 Mutations in the Cacnl1a4 calcium channel gene are associated with seizures, cerebellar degeneration, and ataxia in tottering and leaner mutant mice Mamm Genome 8 113 120
    • (1997) Mamm Genome , vol.8 , pp. 113-120
    • Doyle, J.1    Ren, X.2    Lennon, G.3    Stubbs, L.4
  • 106
    • 20444380384 scopus 로고    scopus 로고
    • Function and dysfunction of synaptic calcium channels: Insights from mouse models
    • D Pietrobon 2005 Function and dysfunction of synaptic calcium channels: insights from mouse models Curr Opin Neurobiol 15 257 265
    • (2005) Curr Opin Neurobiol , vol.15 , pp. 257-265
    • Pietrobon, D.1
  • 107
    • 0035940018 scopus 로고    scopus 로고
    • Ectopic expression of tyrosine hydroxylase in Zebrin II immunoreactive Purkinje cells in the cerebellum of the ataxic mutant mouse, pogo
    • YG Jeong MK Kim R Hawkes 2001 Ectopic expression of tyrosine hydroxylase in Zebrin II immunoreactive Purkinje cells in the cerebellum of the ataxic mutant mouse, pogo Brain Res Dev Brain Res 129 201 209
    • (2001) Brain Res Dev Brain Res , vol.129 , pp. 201-209
    • Jeong, Y.G.1    Kim, M.K.2    Hawkes, R.3
  • 108
    • 0026026842 scopus 로고
    • Tottering and leaner mutations perturb transient developmental expression of tyrosine hydroxylase in embryologically distinct Purkinje cells
    • EJ Hess MC Wilson 1991 Tottering and leaner mutations perturb transient developmental expression of tyrosine hydroxylase in embryologically distinct Purkinje cells Neuron 6 123 132
    • (1991) Neuron , vol.6 , pp. 123-132
    • Hess, E.J.1    Wilson, M.C.2
  • 109
    • 0344299283 scopus 로고    scopus 로고
    • Morphologic investigation of rolling mouse Nagoya (tg(rol)/tg(rol)) cerebellar Purkinje cells: An ataxic mutant, revisited
    • IJ Rhyu S Oda CS Uhm H Kim YS Suh LC Abbott 1999 Morphologic investigation of rolling mouse Nagoya (tg(rol)/tg(rol)) cerebellar Purkinje cells: an ataxic mutant, revisited Neurosci Lett 266 49 52
    • (1999) Neurosci Lett , vol.266 , pp. 49-52
    • Rhyu, I.J.1    Oda, S.2    Uhm, C.S.3    Kim, H.4    Suh, Y.S.5    Abbott, L.C.6
  • 110
    • 0032861945 scopus 로고    scopus 로고
    • Abnormal expression of tyrosine hydroxylase immunoreactivity in Purkinje cells precedes the onset of ataxia in dilute-lethal mice
    • K Sawada S Komatsu H Haga S Oda Y Fukui 1999 Abnormal expression of tyrosine hydroxylase immunoreactivity in Purkinje cells precedes the onset of ataxia in dilute-lethal mice Brain Res 844 188 191
    • (1999) Brain Res , vol.844 , pp. 188-191
    • Sawada, K.1    Komatsu, S.2    Haga, H.3    Oda, S.4    Fukui, Y.5
  • 111
    • 0029451701 scopus 로고
    • Aldolase C/zebrin II and the regionalization of the cerebellum
    • R Hawkes KJ Herrup 1995 Aldolase C/zebrin II and the regionalization of the cerebellum Mol Neurosci 6 147 158
    • (1995) Mol Neurosci , vol.6 , pp. 147-158
    • Hawkes, R.1    Herrup, K.J.2
  • 112
    • 69549112881 scopus 로고    scopus 로고
    • The ataxic Cacna1a-mutant mouse rolling Nagoya: An overview of neuromorphological and electrophysiological findings
    • doi: 10.1007/s12311-009-0117-5
    • Plomp JJ, van den Maagdenberg AMJ, Kaja S (2009) The ataxic Cacna1a-mutant mouse rolling Nagoya: an overview of neuromorphological and electrophysiological findings. Cerebellum. doi: 10.1007/s12311-009-0117-5
    • (2009) Cerebellum
    • Plomp, J.J.1    Van Den Maagdenberg, A.M.J.2    Kaja, S.3
  • 113
    • 0015678982 scopus 로고
    • The observation of rolling mouse Nagoya (rol), a new neurological mutant, and its maintenance
    • S Oda 1973 The observation of rolling mouse Nagoya (rol), a new neurological mutant, and its maintenance Jikken Dobutsu 22 281 288
    • (1973) Jikken Dobutsu , vol.22 , pp. 281-288
    • Oda, S.1
  • 115
    • 0036553669 scopus 로고    scopus 로고
    • Insights from mouse models of absence epilepsy into Ca2+ channel physiology and disease etiology
    • R Felix 2002 Insights from mouse models of absence epilepsy into Ca2+ channel physiology and disease etiology Cell Mol Neurobiol 22 103 120
    • (2002) Cell Mol Neurobiol , vol.22 , pp. 103-120
    • Felix, R.1
  • 116
    • 34447333096 scopus 로고    scopus 로고
    • Spinocerebellar ataxias: An update
    • BW Soong HL Paulson 2007 Spinocerebellar ataxias: an update Curr Opin Neurol 20 438 446
    • (2007) Curr Opin Neurol , vol.20 , pp. 438-446
    • Soong, B.W.1    Paulson, H.L.2
  • 117
    • 69549086398 scopus 로고    scopus 로고
    • The robotic mouse: Understanding the role of AF4, a cofactor of transcriptional elongation and chromatin remodelling, in Purkinje cell function
    • doi: 10.1007/s12311-009-0101-0
    • Bitoun E, Davies KE. The robotic mouse: understanding the role of AF4, a cofactor of transcriptional elongation and chromatin remodelling, in Purkinje cell function. Cerebellum. doi: 10.1007/s12311-009-0101-0
    • Cerebellum
    • Bitoun, E.1    Davies, K.E.2
  • 118
    • 34250157036 scopus 로고    scopus 로고
    • Behavioural characterisation of the robotic mouse mutant
    • PL Oliver DA Keays KE Davies 2007 Behavioural characterisation of the robotic mouse mutant Behav Brain Res 181 239 247
    • (2007) Behav Brain Res , vol.181 , pp. 239-247
    • Oliver, P.L.1    Keays, D.A.2    Davies, K.E.3
  • 119
    • 0037337235 scopus 로고    scopus 로고
    • A mutation in Af4 is predicted to cause cerebellar ataxia and cataracts in the robotic mouse
    • AM Isaacs PL Oliver EL Jones A Jeans A Potter BH Hovik, et al. 2003 A mutation in Af4 is predicted to cause cerebellar ataxia and cataracts in the robotic mouse J Neurosci 23 1631 1637
    • (2003) J Neurosci , vol.23 , pp. 1631-1637
    • Isaacs, A.M.1    Oliver, P.L.2    Jones, E.L.3    Jeans, A.4    Potter, A.5    Hovik, B.H.6
  • 120
    • 5644283146 scopus 로고    scopus 로고
    • Cellular and molecular control of dendritic growth and development of cerebellar Purkinje cells
    • JP Kapfhammer 2004 Cellular and molecular control of dendritic growth and development of cerebellar Purkinje cells Prog Histochem Cytochem 39 131 182
    • (2004) Prog Histochem Cytochem , vol.39 , pp. 131-182
    • Kapfhammer, J.P.1
  • 121
    • 0034662218 scopus 로고    scopus 로고
    • Altered lymphoid development in mice deficient for the mAF4 proto-oncogene
    • P Isnard N Core P Naquet M Djabali 2000 Altered lymphoid development in mice deficient for the mAF4 proto-oncogene Blood 96 705 710
    • (2000) Blood , vol.96 , pp. 705-710
    • Isnard, P.1    Core, N.2    Naquet, P.3    Djabali, M.4
  • 123
    • 0029616201 scopus 로고
    • Domains with transcriptional regulatory activity within the ALL1 and AF4 proteins involved in acute leukemia
    • R Prasad T Yano C Sorio T Nakamura R Rallapalli Y Gu, et al. 1995 Domains with transcriptional regulatory activity within the ALL1 and AF4 proteins involved in acute leukemia Proc Natl Acad Sci U S A 92 12160 12164
    • (1995) Proc Natl Acad Sci U S A , vol.92 , pp. 12160-12164
    • Prasad, R.1    Yano, T.2    Sorio, C.3    Nakamura, T.4    Rallapalli, R.5    Gu, Y.6
  • 124
    • 0030137717 scopus 로고    scopus 로고
    • Identification of the gene FMR2, associated with FRAXE mental retardation
    • J Gecz AK Gedeon GR Sutherland JC Mulley 1996 Identification of the gene FMR2, associated with FRAXE mental retardation Nat Genet 13 105 108
    • (1996) Nat Genet , vol.13 , pp. 105-108
    • Gecz, J.1    Gedeon, A.K.2    Sutherland, G.R.3    Mulley, J.C.4
  • 125
    • 0036549187 scopus 로고    scopus 로고
    • Impaired conditioned fear and enhanced long-term potentiation in Fmr2 knock-out mice
    • Y Gu KL McIlwain EJ Weeber T Yamagata B Xu BA Antalffy, et al. 2002 Impaired conditioned fear and enhanced long-term potentiation in Fmr2 knock-out mice J Neurosci 22 2753 2763
    • (2002) J Neurosci , vol.22 , pp. 2753-2763
    • Gu, Y.1    McIlwain, K.L.2    Weeber, E.J.3    Yamagata, T.4    Xu, B.5    Antalffy, B.A.6
  • 126
    • 69549118449 scopus 로고    scopus 로고
    • Cerebellum-related characteristics of Scn8a-mutant mice
    • doi: 10.1007/s12311-009-0110-z
    • Chen K, Godfrey DA, Ilyas O, Xu J, Preston TW. Cerebellum-related characteristics of Scn8a-mutant mice. Cerebellum. doi: 10.1007/s12311-009-0110-z
    • Cerebellum
    • Chen, K.1    Godfrey, D.A.2    Ilyas, O.3    Xu, J.4    Preston, T.W.5
  • 128
    • 33745281204 scopus 로고    scopus 로고
    • Heterozygosity for a protein truncation mutation of sodium channel Scn8a in a patient with cerebellar atrophy, ataxia and mental retardation
    • MM Trudeau JC Dalton JW Day LPW Ranum MH Meisler 2006 Heterozygosity for a protein truncation mutation of sodium channel Scn8a in a patient with cerebellar atrophy, ataxia and mental retardation J Med Genet 43 527 530
    • (2006) J Med Genet , vol.43 , pp. 527-530
    • Trudeau, M.M.1    Dalton, J.C.2    Day, J.W.3    Ranum, L.P.W.4    Meisler, M.H.5
  • 130
    • 0030015959 scopus 로고    scopus 로고
    • Mutation detection in the med and medJ alleles of the sodium channel Scn8a
    • DC Kohrman JB Harris MH Meisler 1996 Mutation detection in the med and medJ alleles of the sodium channel Scn8a J Biol Chem 271 17576 17581
    • (1996) J Biol Chem , vol.271 , pp. 17576-17581
    • Kohrman, D.C.1    Harris, J.B.2    Meisler, M.H.3
  • 133
    • 50449107176 scopus 로고    scopus 로고
    • The undertow of sulfur metabolism on glutamatergic neurotransmission
    • R Banerjee V Vitvitsky SK Garg 2008 The undertow of sulfur metabolism on glutamatergic neurotransmission Trends Biochem Sci 33 413 419
    • (2008) Trends Biochem Sci , vol.33 , pp. 413-419
    • Banerjee, R.1    Vitvitsky, V.2    Garg, S.K.3
  • 134
    • 69549088145 scopus 로고    scopus 로고
    • Bergmann glial S100B activates myo-inositol monophosphatase 1 and co-localizes to Purkinje cell vacuoles in SCA1 transgenic mice
    • doi: 10.1007/s12311-009-0125-5
    • Vig PJS, Shao Q, Subramony SH, Lopez ME, Safaya E. Bergmann glial S100B activates myo-inositol monophosphatase 1 and co-localizes to Purkinje cell vacuoles in SCA1 transgenic mice. Cerebellum. doi: 10.1007/s12311-009-0125-5
    • Cerebellum
    • Vig, P.J.S.1    Shao, Q.2    Subramony, S.H.3    Lopez, M.E.4    Safaya, E.5
  • 135
  • 137
    • 65849514220 scopus 로고    scopus 로고
    • SCA3: Neurological features, pathogenesis and animal models
    • O Riess U Rüb A Pastore P Bauer L Schöls 2008 SCA3: neurological features, pathogenesis and animal models Cerebellum 7 2 125 137
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 125-137
    • Riess, O.1    Rüb, U.2    Pastore, A.3    Bauer, P.4    Schöls, L.5
  • 138
    • 29044445801 scopus 로고    scopus 로고
    • Polyglutamine-expanded ataxin-7 activates mitochondrial apoptotic pathway of cerebellar neurons by upregulating Bax and downregulating Bcl-x(L)
    • HL Wang TH Yeh AH Chou YL Kuo LJ Luo CY He PC Huang AH Li 2006 Polyglutamine-expanded ataxin-7 activates mitochondrial apoptotic pathway of cerebellar neurons by upregulating Bax and downregulating Bcl-x(L) Cell Signal 18 4 541 552
    • (2006) Cell Signal , vol.18 , Issue.4 , pp. 541-552
    • Wang, H.L.1    Yeh, T.H.2    Chou, A.H.3    Kuo, Y.L.4    Luo, L.J.5    He, C.Y.6    Huang, P.C.7    Li, A.H.8
  • 139
    • 65849122227 scopus 로고    scopus 로고
    • Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration
    • GA Garden AR La Spada 2008 Molecular pathogenesis and cellular pathology of spinocerebellar ataxia type 7 neurodegeneration Cerebellum 7 2 138 149
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 138-149
    • Garden, G.A.1    La Spada, A.R.2
  • 140
    • 65849116838 scopus 로고    scopus 로고
    • Spinocerebellar ataxia 17 (SCA17) and Huntington's disease-like 4 (HDL4)
    • G Stevanin A Brice 2008 Spinocerebellar ataxia 17 (SCA17) and Huntington's disease-like 4 (HDL4) Cerebellum 7 2 170 178
    • (2008) Cerebellum , vol.7 , Issue.2 , pp. 170-178
    • Stevanin, G.1    Brice, A.2
  • 141
    • 36949022900 scopus 로고    scopus 로고
    • CAG repeat disorder models and human neuropathology: Similarities and differences
    • M Yamada T Sato S Tsuji H Takahashi 2008 CAG repeat disorder models and human neuropathology: similarities and differences Acta Neuropathol 115 1 71 86
    • (2008) Acta Neuropathol , vol.115 , Issue.1 , pp. 71-86
    • Yamada, M.1    Sato, T.2    Tsuji, S.3    Takahashi, H.4
  • 142
    • 74249092236 scopus 로고    scopus 로고
    • Mouse models of fragile X-associated tremor ataxia
    • Berman RF, Willemsen R (2009) Mouse models of fragile X-associated tremor ataxia. J Investig Med (in press)
    • (2009) J Investig Med (In Press)
    • Berman, R.F.1
  • 144
    • 53849113948 scopus 로고    scopus 로고
    • Comparative evaluation of the cerebral and cerebellar white matter development in pediatric age group using quantitative diffusion tensor imaging
    • S Saksena N Husain GK Malik R Trivedi M Sarma RS Rathore CM Pandey RK Gupta 2008 Comparative evaluation of the cerebral and cerebellar white matter development in pediatric age group using quantitative diffusion tensor imaging Cerebellum 7 3 392 400
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 392-400
    • Saksena, S.1    Husain, N.2    Malik, G.K.3    Trivedi, R.4    Sarma, M.5    Rathore, R.S.6    Pandey, C.M.7    Gupta, R.K.8
  • 147
    • 0019771755 scopus 로고
    • Five new mutations in the Syrian hamsters: Fur loss, fur deficiency, juvenile gray, ashen, and quaking
    • JS Peterson CT de Groot CH Yoon 1981 Five new mutations in the Syrian hamsters: fur loss, fur deficiency, juvenile gray, ashen, and quaking J Hered 72 445 446
    • (1981) J Hered , vol.72 , pp. 445-446
    • Peterson, J.S.1    De Groot, C.T.2    Yoon, C.H.3
  • 148
    • 69549121761 scopus 로고    scopus 로고
    • The ataxic Syrian Hamster: An animal model homologous to the pcd mutant mouse?
    • doi: 10.1007/s12311-009-0113-9
    • Akita K, Arai S (2009) The ataxic Syrian Hamster: an animal model homologous to the pcd mutant mouse? Cerebellum. doi: 10.1007/s12311-009-0113-9
    • (2009) Cerebellum
    • Akita, K.1    Arai, S.2
  • 149
    • 2042422363 scopus 로고
    • Purkinje cell degeneration, a new neurological mutation in the mouse
    • RJ Mullen EM Eicher RL Sidman 1976 Purkinje cell degeneration, a new neurological mutation in the mouse Proc Nat Acad Sci U S A 73 208 212
    • (1976) Proc Nat Acad Sci U S A , vol.73 , pp. 208-212
    • Mullen, R.J.1    Eicher, E.M.2    Sidman, R.L.3
  • 151
    • 0029129244 scopus 로고
    • Graft-induced restoration of function in hereditary cerebellar ataxia
    • LC Triarhou W Zhang WH Lee 1995 Graft-induced restoration of function in hereditary cerebellar ataxia NeuroReport 6 1827 1832
    • (1995) NeuroReport , vol.6 , pp. 1827-1832
    • Triarhou, L.C.1    Zhang, W.2    Lee, W.H.3
  • 153
    • 33847661633 scopus 로고    scopus 로고
    • The Purkinje cell degeneration (pcd) mouse: An unexpected molecular link between neuronal degeneration and regeneration
    • T Wang JI Morgan 2007 The Purkinje cell degeneration (pcd) mouse: an unexpected molecular link between neuronal degeneration and regeneration Brain Res 1140 26 40
    • (2007) Brain Res , vol.1140 , pp. 26-40
    • Wang, T.1    Morgan, J.I.2
  • 154
    • 0032718963 scopus 로고    scopus 로고
    • Alteration of IGF system gene expression during the postnatal development of pcd mice
    • W Zang B Ghetti XL Yang WH Lee 1999 Alteration of IGF system gene expression during the postnatal development of pcd mice J Endocrinol 163 191 198
    • (1999) J Endocrinol , vol.163 , pp. 191-198
    • Zang, W.1    Ghetti, B.2    Yang, X.L.3    Lee, W.H.4
  • 155
    • 0000314663 scopus 로고
    • A quantitative study of the granule cells in Purkinje cell degeneration (pcd) mutant
    • LC Triarhou J Norton CJ Alyea B Ghetti 1985 A quantitative study of the granule cells in Purkinje cell degeneration (pcd) mutant Ann Neurol 18 146
    • (1985) Ann Neurol , vol.18 , pp. 146
    • Triarhou, L.C.1    Norton, J.2    Alyea, C.J.3    Ghetti, B.4
  • 156
    • 0021914258 scopus 로고
    • Degeneration of thalamic neurons in 'Purkinje cell degeneration' mutant mice. I. Distribution of neuron loss
    • DOI 10.1002/cne.902340302
    • S O'Gorman RL Sidman 1985 Degeneration of thalamic neurons in "Purkinje cell degeneration" mutant mice. I. Distribution of neuron loss J Comp Neurol 234 277 297 (Pubitemid 15090221)
    • (1985) Journal of Comparative Neurology , vol.234 , Issue.3 , pp. 277-297
    • O'Gorman, S.1    Sidman, R.L.2
  • 158
    • 0034533467 scopus 로고    scopus 로고
    • Regenerating motor neurons express Nna1, a novel ATP/GTP-binding protein related to zinc carboxypeptidase
    • A Harris JI Morgan M Pecot A Soumare A Osborne HD Soares 2000 Regenerating motor neurons express Nna1, a novel ATP/GTP-binding protein related to zinc carboxypeptidase Mol Cell Neurosci 16 578 596
    • (2000) Mol Cell Neurosci , vol.16 , pp. 578-596
    • Harris, A.1    Morgan, J.I.2    Pecot, M.3    Soumare, A.4    Osborne, A.5    Soares, H.D.6
  • 160
    • 0035971218 scopus 로고    scopus 로고
    • Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice
    • N Sawamura JS Gong WS Garver RA Heidenreich H Ninomiya K Ohno K Yanagisawa M Michikawa 2001 Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice J Biol Chem 276 10314 10319
    • (2001) J Biol Chem , vol.276 , pp. 10314-10319
    • Sawamura, N.1    Gong, J.S.2    Garver, W.S.3    Heidenreich, R.A.4    Ninomiya, H.5    Ohno, K.6    Yanagisawa, K.7    Michikawa, M.8
  • 161
    • 0037340949 scopus 로고    scopus 로고
    • Promotion of tau phosphorylation by MAP kinase Erk1/2 is accompanied by reduced cholesterol level in detergent-insoluble membrane fraction in Niemann-Pick C1-deficient cells
    • N Sawamura JS Gong TY Chang K Yanagisawa M Michikawa 2003 Promotion of tau phosphorylation by MAP kinase Erk1/2 is accompanied by reduced cholesterol level in detergent-insoluble membrane fraction in Niemann-Pick C1-deficient cells J Neurochem 84 5 1086 1096
    • (2003) J Neurochem , vol.84 , Issue.5 , pp. 1086-1096
    • Sawamura, N.1    Gong, J.S.2    Chang, T.Y.3    Yanagisawa, K.4    Michikawa, M.5
  • 162
    • 0036703779 scopus 로고    scopus 로고
    • Deregulation of cdk5, hyperphosphorylation, and cytoskeletal pathology in the Niemann-Pick type C murine model
    • B Bu J Li P Davies I Vincent 2002 Deregulation of cdk5, hyperphosphorylation, and cytoskeletal pathology in the Niemann-Pick type C murine model J Neurosci 22 6515 6525
    • (2002) J Neurosci , vol.22 , pp. 6515-6525
    • Bu, B.1    Li, J.2    Davies, P.3    Vincent, I.4
  • 163
    • 0011749436 scopus 로고
    • A new recessive lethal mutation in mice
    • KB DeOme 1945 A new recessive lethal mutation in mice Univ Calif Publ Zool 53 41 55
    • (1945) Univ Calif Publ Zool , vol.53 , pp. 41-55
    • Deome, K.B.1
  • 166
    • 0024801519 scopus 로고
    • Interspecies genetic complementation analysis of human and sheep fibroblasts with,-galactosidase deficiency
    • AJ Ahern-Rindell RD Murnane DJ Prieur 1989 Interspecies genetic complementation analysis of human and sheep fibroblasts with,-galactosidase deficiency Somat Cell Genet 15 525 533
    • (1989) Somat Cell Genet , vol.15 , pp. 525-533
    • Ahern-Rindell, A.J.1    Murnane, R.D.2    Prieur, D.J.3
  • 169
    • 0026170574 scopus 로고
    • Application of lectin histochemistry and carbohydrate analysis to the characterization of lysosomal storage diseases
    • J Alroy R DeGasperi CD Warren 1991 Application of lectin histochemistry and carbohydrate analysis to the characterization of lysosomal storage diseases Carbohydr Res 213 229 250
    • (1991) Carbohydr Res , vol.213 , pp. 229-250
    • Alroy, J.1    Degasperi, R.2    Warren, C.D.3
  • 171
    • 0020609566 scopus 로고
    • Presence of activator proteins for the enzymic hydrolysis of GM, and GM2 gangliosides in normal human urine
    • YT Li IA Muhiudeen R DeGasperi Y Hirabayashi SC Li 1983 Presence of activator proteins for the enzymic hydrolysis of GM, and GM2 gangliosides in normal human urine Am J Hum Genet 35 629 634
    • (1983) Am J Hum Genet , vol.35 , pp. 629-634
    • Li, Y.T.1    Muhiudeen, I.A.2    Degasperi, R.3    Hirabayashi, Y.4    Li, S.C.5
  • 173
    • 0029353191 scopus 로고
    • Wilson's disease: A new gene and an animal model for an old disease
    • JA Cuthbert 1995 Wilson's disease: a new gene and an animal model for an old disease J Investig Med 43 323 336
    • (1995) J Investig Med , vol.43 , pp. 323-336
    • Cuthbert, J.A.1
  • 174
    • 0028001088 scopus 로고
    • The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene
    • J Wu JR Forbes HS Chen DW Cox 1994 The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene Nat Genet 7 541 545
    • (1994) Nat Genet , vol.7 , pp. 541-545
    • Wu, J.1    Forbes, J.R.2    Chen, H.S.3    Cox, D.W.4
  • 175
  • 177
    • 0029098377 scopus 로고
    • The toxic milk mutation, tx, which results in a condition resembling Wilson disease in humans, is linked to mouse chromosome 8
    • H Rauch AJ Wells 1995 The toxic milk mutation, tx, which results in a condition resembling Wilson disease in humans, is linked to mouse chromosome 8 Genomics 29 551 552
    • (1995) Genomics , vol.29 , pp. 551-552
    • Rauch, H.1    Wells, A.J.2
  • 178
    • 26444432919 scopus 로고    scopus 로고
    • Mitochondrial DNA polymerase gamma is essential for mammalian embryogenesis
    • N Hance MI Ekstrand A Trifunovic 2005 Mitochondrial DNA polymerase gamma is essential for mammalian embryogenesis Hum Mol Genet 14 13 1775 1783
    • (2005) Hum Mol Genet , vol.14 , Issue.13 , pp. 1775-1783
    • Hance, N.1    Ekstrand, M.I.2    Trifunovic, A.3
  • 180
    • 33745712834 scopus 로고    scopus 로고
    • Mice with neuron-specific accumulation of mitochondrial DNA mutations show mood disorder-like phenotypes
    • 523
    • T Kasahara M Kubota T Miyauchi Y Noda A Mouri T Nabeshima T Kato 2006 Mice with neuron-specific accumulation of mitochondrial DNA mutations show mood disorder-like phenotypes Mol Psychiatry 11 6 577 593 523
    • (2006) Mol Psychiatry , vol.11 , Issue.6 , pp. 577-593
    • Kasahara, T.1    Kubota, M.2    Miyauchi, T.3    Noda, Y.4    Mouri, A.5    Nabeshima, T.6    Kato, T.7
  • 182
    • 4844221895 scopus 로고    scopus 로고
    • Lysosomal sulfatide storage in the brain of arylsulfatase A-deficient mice: Cellular alterations and topographic distribution
    • D Wittke D Hartmann V Gieselmann R Lüllmann-Rauch 2004 Lysosomal sulfatide storage in the brain of arylsulfatase A-deficient mice: cellular alterations and topographic distribution Acta Neuropathol (Berl) 108 261 271
    • (2004) Acta Neuropathol (Berl) , vol.108 , pp. 261-271
    • Wittke, D.1    Hartmann, D.2    Gieselmann, V.3    Lüllmann-Rauch, R.4
  • 184
    • 4644293303 scopus 로고    scopus 로고
    • Disruption of ceruloplasmin and hephaestin in mice causes retinal iron overload and retinal degeneration with features of age-related macular degeneration
    • P Hahn Y Qian T Dentchev L Chen J Beard ZL Harris JL Dunaief 2004 Disruption of ceruloplasmin and hephaestin in mice causes retinal iron overload and retinal degeneration with features of age-related macular degeneration Proc Natl Acad Sci U S A 101 38 13850 13855
    • (2004) Proc Natl Acad Sci U S A , vol.101 , Issue.38 , pp. 13850-13855
    • Hahn, P.1    Qian, Y.2    Dentchev, T.3    Chen, L.4    Beard, J.5    Harris, Z.L.6    Dunaief, J.L.7
  • 188
    • 0034194228 scopus 로고    scopus 로고
    • (Over)correction of FMR1 deficiency with YAC transgenics: Behavioral and physical features
    • AM Peier KL McIlwain A Kenneson ST Warren R Paylor DL Nelson 2000 (Over)correction of FMR1 deficiency with YAC transgenics: behavioral and physical features Hum Mol Genet 9 1145 1159
    • (2000) Hum Mol Genet , vol.9 , pp. 1145-1159
    • Peier, A.M.1    Kenneson, A.2    Warren, S.T.3    Paylor, R.4    Nelson, D.L.5
  • 190
    • 0036501313 scopus 로고    scopus 로고
    • Late onset neurological phenotype of the X-ALD gene inactivation in mice: A mouse model for adrenomyeloneuropathy
    • A Pujol C Hindelang N Callizot U Bartsch M Schachner JL Mandel 2002 Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy Hum Mol Genet 11 499 505
    • (2002) Hum Mol Genet , vol.11 , pp. 499-505
    • Pujol, A.1    Hindelang, C.2    Callizot, N.3    Bartsch, U.4    Schachner, M.5    Mandel, J.L.6
  • 195
    • 34548675598 scopus 로고    scopus 로고
    • Magnetic resonance imaging (MRI) findings and neuropsychological sequelae in children after severe traumatic brain injury: The role of cerebellar lesion
    • LW Braga LN Souza YJ Najjar G Dellatolas 2007 Magnetic resonance imaging (MRI) findings and neuropsychological sequelae in children after severe traumatic brain injury: the role of cerebellar lesion J Child Neurol 22 9 1084 1089
    • (2007) J Child Neurol , vol.22 , Issue.9 , pp. 1084-1089
    • Braga, L.W.1    Souza, L.N.2    Najjar, Y.J.3    Dellatolas, G.4
  • 201
    • 0037240606 scopus 로고    scopus 로고
    • Nitric oxide levels in rat cortex, hippocampus, cerebellum, and brainstem after impact acceleration head injury
    • S Tuzgen N Tanriover M Uzan E Tureci T Tanriverdi K Gumustas, et al. 2003 Nitric oxide levels in rat cortex, hippocampus, cerebellum, and brainstem after impact acceleration head injury Neurol Res 25 1 31 34
    • (2003) Neurol Res , vol.25 , Issue.1 , pp. 31-34
    • Tuzgen, S.1    Tanriover, N.2    Uzan, M.3    Tureci, E.4    Tanriverdi, T.5    Gumustas, K.6
  • 202
    • 0035083911 scopus 로고    scopus 로고
    • A new model for diffuse brain injury by rotational acceleration: I model, gross appearance, and astrocytosis
    • E Gutierrez Y Huang K Haglid F Bao HA Hansson A Hamberger, et al. 2001 A new model for diffuse brain injury by rotational acceleration: I model, gross appearance, and astrocytosis J Neurotrauma 18 3 247 257
    • (2001) J Neurotrauma , vol.18 , Issue.3 , pp. 247-257
    • Gutierrez, E.1    Huang, Y.2    Haglid, K.3    Bao, F.4    Hansson, H.A.5    Hamberger, A.6
  • 203
    • 0035079796 scopus 로고    scopus 로고
    • A new model for diffuse brain injury by rotational acceleration: II. Effects on extracellular glutamate, intracranial pressure, and neuronal apoptosis
    • M Runnerstam F Bao Y Huang J Shi E Gutierrez A Hamberger, et al. 2001 A new model for diffuse brain injury by rotational acceleration: II. Effects on extracellular glutamate, intracranial pressure, and neuronal apoptosis J Neurotrauma 18 3 259 273
    • (2001) J Neurotrauma , vol.18 , Issue.3 , pp. 259-273
    • Runnerstam, M.1    Bao, F.2    Huang, Y.3    Shi, J.4    Gutierrez, E.5    Hamberger, A.6
  • 204
    • 4043153466 scopus 로고    scopus 로고
    • Presynaptic excitability as a potential target for the treatment of the traumatic cerebellum
    • J Ai A Baker 2004 Presynaptic excitability as a potential target for the treatment of the traumatic cerebellum Pharmacology 71 4 192 198
    • (2004) Pharmacology , vol.71 , Issue.4 , pp. 192-198
    • Ai, J.1    Baker, A.2
  • 205
    • 0028085854 scopus 로고
    • Lack of Purkinje cell loss in adult rat cerebellum following protracted axotomy: Degenerative changes and regenerative attempts of the severed axons
    • I Dusart C Sotelo 1994 Lack of Purkinje cell loss in adult rat cerebellum following protracted axotomy: degenerative changes and regenerative attempts of the severed axons J Comp Neurol 347 2 211 232
    • (1994) J Comp Neurol , vol.347 , Issue.2 , pp. 211-232
    • Dusart, I.1    Sotelo, C.2
  • 207
    • 2642510627 scopus 로고    scopus 로고
    • Reparative mechanisms in the cerebellar cortex
    • D Carulli A Buffo P Strata 2004 Reparative mechanisms in the cerebellar cortex Prog Neurobiol 72 6 373 398
    • (2004) Prog Neurobiol , vol.72 , Issue.6 , pp. 373-398
    • Carulli, D.1    Buffo, A.2    Strata, P.3
  • 208
    • 33745865919 scopus 로고    scopus 로고
    • The strange case of Purkinje axon regeneration and plasticity
    • F Rossi S Gianola L Corvetti 2006 The strange case of Purkinje axon regeneration and plasticity Cerebellum 5 2 174 182
    • (2006) Cerebellum , vol.5 , Issue.2 , pp. 174-182
    • Rossi, F.1    Gianola, S.2    Corvetti, L.3
  • 209
    • 0036913056 scopus 로고    scopus 로고
    • Neuroprotection and traumatic brain injury: Theoretical option or realistic proposition
    • AI Faden 2002 Neuroprotection and traumatic brain injury: theoretical option or realistic proposition Curr Opin Neurol 15 707 712
    • (2002) Curr Opin Neurol , vol.15 , pp. 707-712
    • Faden, A.I.1
  • 211
    • 53849100646 scopus 로고    scopus 로고
    • Minocycline attenuates microglial activation but fails to mitigate degeneration in inferior olive and pontine nuclei after focal cerebellar lesion
    • MT Viscomi L Latini F Florenzano G Bernardi M Molinari 2008 Minocycline attenuates microglial activation but fails to mitigate degeneration in inferior olive and pontine nuclei after focal cerebellar lesion Cerebellum 7 401 405
    • (2008) Cerebellum , vol.7 , pp. 401-405
    • Viscomi, M.T.1    Latini, L.2    Florenzano, F.3    Bernardi, G.4    Molinari, M.5
  • 212
    • 0029744418 scopus 로고    scopus 로고
    • Neuroprotective effects of human recombinant interleukin-1 receptor antagonist in focal cerebral ischaemia in the rat
    • SA Loddick NJ Rothwell 1996 Neuroprotective effects of human recombinant interleukin-1 receptor antagonist in focal cerebral ischaemia in the rat J Cereb Blood Flow Metab 16 932 940
    • (1996) J Cereb Blood Flow Metab , vol.16 , pp. 932-940
    • Loddick, S.A.1    Rothwell, N.J.2
  • 213
    • 33645123006 scopus 로고    scopus 로고
    • Minocycline attenuates microglial activation but fails to mitigate striatal dopaminergic neurotoxicity: Role of tumor necrosis factor-alpha
    • K Sriram DB Miller JP O'Callaghan 2006 Minocycline attenuates microglial activation but fails to mitigate striatal dopaminergic neurotoxicity: role of tumor necrosis factor-alpha J Neurochem 96 706 718
    • (2006) J Neurochem , vol.96 , pp. 706-718
    • Sriram, K.1    Miller, D.B.2    O'Callaghan, J.P.3
  • 214
    • 58149174089 scopus 로고    scopus 로고
    • The evaluation of effect of alpha-lipoic acid and vitamin e on the lipid peroxidation, gamma-amino butyric acid and serotonin level in the brain of mice (Mus musculus) acutely intoxicated with lindane
    • R Bist DK Bhatt 2009 The evaluation of effect of alpha-lipoic acid and vitamin E on the lipid peroxidation, gamma-amino butyric acid and serotonin level in the brain of mice (Mus musculus) acutely intoxicated with lindane J Neurol Sci 276 1-2 99 102
    • (2009) J Neurol Sci , vol.276 , Issue.12 , pp. 99-102
    • Bist, R.1    Bhatt, D.K.2
  • 215
  • 216
    • 46249132336 scopus 로고    scopus 로고
    • Ethanol-related increases in degenerating bodies in the Purkinje neuron dendrites of aging rats
    • CA Dlugos 2008 Ethanol-related increases in degenerating bodies in the Purkinje neuron dendrites of aging rats Brain Res 1221 98 107
    • (2008) Brain Res , vol.1221 , pp. 98-107
    • Dlugos, C.A.1
  • 217
    • 56249105771 scopus 로고    scopus 로고
    • The protective effect of neuronal nitric oxide synthase (nNOS) against alcohol toxicity depends upon the NO-cGMP-PKG pathway and NF-kappaB
    • DJ Bonthius NE Bonthius S Li B Karacay 2008 The protective effect of neuronal nitric oxide synthase (nNOS) against alcohol toxicity depends upon the NO-cGMP-PKG pathway and NF-kappaB Neurotoxicology 29 6 1080 1091
    • (2008) Neurotoxicology , vol.29 , Issue.6 , pp. 1080-1091
    • Bonthius, D.J.1    Bonthius, N.E.2    Li, S.3    Karacay, B.4
  • 218
    • 69549105728 scopus 로고    scopus 로고
    • Cuprizone treatment induces distinct demyelination, astrocytosis, and microglia cell invasion or proliferation in the mouse cerebellum
    • doi: 10.1007/s12311-009-0099-3
    • Groebe A, Clarner T, Baumgartner W, Dang J, Beyer C, Kipp M. Cuprizone treatment induces distinct demyelination, astrocytosis, and microglia cell invasion or proliferation in the mouse cerebellum. Cerebellum. doi: 10.1007/s12311-009-0099-3
    • Cerebellum
    • Groebe, A.1    Clarner, T.2    Baumgartner, W.3    Dang, J.4    Beyer, C.5    Kipp, M.6
  • 220
    • 54249124952 scopus 로고    scopus 로고
    • Remyelination in the CNS: From biology to therapy
    • RJ Franklin C French-Constant 2008 Remyelination in the CNS: from biology to therapy Nat Rev Neurosci 9 11 839 855
    • (2008) Nat Rev Neurosci , vol.9 , Issue.11 , pp. 839-855
    • Franklin, R.J.1    French-Constant, C.2
  • 221
    • 0035096330 scopus 로고    scopus 로고
    • Heterogeneity of multiple sclerosis pathogenesis: Implications for diagnosis and therapy
    • H Lassmann W Bruck C Lucchinetti 2001 Heterogeneity of multiple sclerosis pathogenesis: implications for diagnosis and therapy Trends Mol Med 7 3 115 121
    • (2001) Trends Mol Med , vol.7 , Issue.3 , pp. 115-121
    • Lassmann, H.1    Bruck, W.2    Lucchinetti, C.3
  • 222
    • 0035198691 scopus 로고    scopus 로고
    • The neurotoxicant, cuprizone, as a model to study demyelination and remyelination in the central nervous system
    • GK Matsushima P Morell 2001 The neurotoxicant, cuprizone, as a model to study demyelination and remyelination in the central nervous system Brain Pathol 11 1 107 116
    • (2001) Brain Pathol , vol.11 , Issue.1 , pp. 107-116
    • Matsushima, G.K.1    Morell, P.2
  • 223
    • 49849098249 scopus 로고    scopus 로고
    • Brain-region-specific astroglial responses in vitro after LPS exposure
    • M Kipp A Norkute S Johann L Lorenz A Braun A Hieble, et al. 2008 Brain-region-specific astroglial responses in vitro after LPS exposure J Mol Neurosci 35 2 235 243
    • (2008) J Mol Neurosci , vol.35 , Issue.2 , pp. 235-243
    • Kipp, M.1    Norkute, A.2    Johann, S.3    Lorenz, L.4    Braun, A.5    Hieble, A.6
  • 224
    • 42949110235 scopus 로고    scopus 로고
    • Expression of enzymes involved in the prostanoid metabolism by cortical astrocytes after LPS-induced inflammation
    • S Johann E Kampmann B Denecke S Arnold M Kipp J Mey, et al. 2008 Expression of enzymes involved in the prostanoid metabolism by cortical astrocytes after LPS-induced inflammation J Mol Neurosci 34 2 177 185
    • (2008) J Mol Neurosci , vol.34 , Issue.2 , pp. 177-185
    • Johann, S.1    Kampmann, E.2    Denecke, B.3    Arnold, S.4    Kipp, M.5    Mey, J.6
  • 226
    • 33751012093 scopus 로고    scopus 로고
    • Carbenoxolone and mefloquine suppress tremor in the harmaline mouse model of essential tremor
    • FC Martin A Handforth 2006 Carbenoxolone and mefloquine suppress tremor in the harmaline mouse model of essential tremor Mov Disord 21 10 1641 1649
    • (2006) Mov Disord , vol.21 , Issue.10 , pp. 1641-1649
    • Martin, F.C.1    Handforth, A.2
  • 229
    • 67651173041 scopus 로고    scopus 로고
    • Identification of a thyroid hormone response element in the mouse Kruppel-like factor 9 gene to explain its postnatal expression in the brain
    • RJ Denver KE Williamson 2009 Identification of a thyroid hormone response element in the mouse Kruppel-like factor 9 gene to explain its postnatal expression in the brain Endocrinology 150 8 3935 3943
    • (2009) Endocrinology , vol.150 , Issue.8 , pp. 3935-3943
    • Denver, R.J.1    Williamson, K.E.2
  • 230
    • 53849144675 scopus 로고    scopus 로고
    • Hormonal disruption in cerebellar development by hydroxylated polychlorinated biphenyls
    • J Kimura-Kuroda I Nagata Y Kuroda 2008 Hormonal disruption in cerebellar development by hydroxylated polychlorinated biphenyls Cerebellum 7 3 500 501
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 500-501
    • Kimura-Kuroda, J.1    Nagata, I.2    Kuroda, Y.3
  • 231
    • 66849099394 scopus 로고    scopus 로고
    • Retinoid-related orphan receptors (RORs): Critical roles in development, immunity, circadian rhythm, and cellular metabolism
    • AM Jetten 2009 Retinoid-related orphan receptors (RORs): critical roles in development, immunity, circadian rhythm, and cellular metabolism Nucl Recept Signal 7 e003
    • (2009) Nucl Recept Signal , vol.7 , pp. 003
    • Jetten, A.M.1
  • 233
    • 84887212627 scopus 로고    scopus 로고
    • Identification of thyroid hormone receptor binding sites and target genes using ChIP-on-chip in developing mouse cerebellum
    • H Dong CL Yauk A Rowan-Carroll SH You RT Zoeller I Lambert MG Wade 2009 Identification of thyroid hormone receptor binding sites and target genes using ChIP-on-chip in developing mouse cerebellum PLoS ONE 4 2 e4610
    • (2009) PLoS ONE , vol.4 , Issue.2 , pp. 4610
    • Dong, H.1    Yauk, C.L.2    Rowan-Carroll, A.3    You, S.H.4    Zoeller, R.T.5    Lambert, I.6    Wade, M.G.7
  • 235
    • 65449163073 scopus 로고    scopus 로고
    • Identification, biosynthesis, and function of 7alpha-hydroxypregnenolone, a new key neurosteroid controlling locomotor activity, in nonmammalian vertebrates
    • K Tsutsui S Haraguchi K Inoue H Miyabara S Suzuki Y Ogura T Koyama M Matsunaga H Vaudry 2009 Identification, biosynthesis, and function of 7alpha-hydroxypregnenolone, a new key neurosteroid controlling locomotor activity, in nonmammalian vertebrates Ann N Y Acad Sci 1163 308 315
    • (2009) Ann N y Acad Sci , vol.1163 , pp. 308-315
    • Tsutsui, K.1    Haraguchi, S.2    Inoue, K.3    Miyabara, H.4    Suzuki, S.5    Ogura, Y.6    Koyama, T.7    Matsunaga, M.8    Vaudry, H.9
  • 236
    • 66449134231 scopus 로고    scopus 로고
    • Expression, localization and possible actions of 25-Dx, a membrane associated putative progesterone-binding protein, in the developing Purkinje cell of the cerebellum: A new insight into the biosynthesis, metabolism and multiple actions of progesterone as a neurosteroid
    • H Sakamoto K Ukena M Kawata K Tsutsui 2008 Expression, localization and possible actions of 25-Dx, a membrane associated putative progesterone-binding protein, in the developing Purkinje cell of the cerebellum: a new insight into the biosynthesis, metabolism and multiple actions of progesterone as a neurosteroid Cerebellum 7 1 18 25
    • (2008) Cerebellum , vol.7 , Issue.1 , pp. 18-25
    • Sakamoto, H.1    Ukena, K.2    Kawata, M.3    Tsutsui, K.4
  • 237
    • 53849100036 scopus 로고    scopus 로고
    • Neurosteroid synthesis and action in the cerebellum during development
    • K Tsutsui 2008 Neurosteroid synthesis and action in the cerebellum during development Cerebellum 7 3 502 504
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 502-504
    • Tsutsui, K.1
  • 238
  • 239
    • 53849125009 scopus 로고    scopus 로고
    • The gonadotropin-releasing hormone type i receptor is expressed in the mouse cerebellum
    • AJ Albertson H Talbott Q Wang D Jensen DC Skinner 2008 The gonadotropin-releasing hormone type I receptor is expressed in the mouse cerebellum Cerebellum 7 3 379 384
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 379-384
    • Albertson, A.J.1    Talbott, H.2    Wang, Q.3    Jensen, D.4    Skinner, D.C.5
  • 240
    • 53849146052 scopus 로고    scopus 로고
    • Brain development, environment and sex: What can we learn from studying graviperception, gravitransduction and the gravireaction of the developing CNS to altered gravity?
    • EM Sajdel-Sulkowska 2008 Brain development, environment and sex: what can we learn from studying graviperception, gravitransduction and the gravireaction of the developing CNS to altered gravity? Cerebellum 7 3 223 239
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 223-239
    • Sajdel-Sulkowska, E.M.1
  • 241
    • 0031598254 scopus 로고    scopus 로고
    • An age-dependent sensitivity of the roll-induced vestibuloocular reflex to hypergravity exposure of several days in an amphibian (Xenopus laevis)
    • CE Sebastian K Pfau ER Horn 1998 An age-dependent sensitivity of the roll-induced vestibuloocular reflex to hypergravity exposure of several days in an amphibian (Xenopus laevis) Acta Astronaut 42 1-8 419 430
    • (1998) Acta Astronaut , vol.42 , Issue.18 , pp. 419-430
    • Sebastian, C.E.1    Pfau, K.2    Horn, E.R.3
  • 242
    • 0036698239 scopus 로고    scopus 로고
    • Adaptation of the macular vestibuloocular reflex to altered gravitational conditions in a fish (Oreochromis mossambicus)
    • E Horn C Sebastian 2002 Adaptation of the macular vestibuloocular reflex to altered gravitational conditions in a fish (Oreochromis mossambicus) Adv Space Res 30 4 711 720
    • (2002) Adv Space Res , vol.30 , Issue.4 , pp. 711-720
    • Horn, E.1    Sebastian, C.2
  • 245
    • 34548438997 scopus 로고    scopus 로고
    • The cerebellum: Comparative and animal studies
    • F Sultan M Glickstein 2007 The cerebellum: comparative and animal studies Cerebellum 6 3 168 176
    • (2007) Cerebellum , vol.6 , Issue.3 , pp. 168-176
    • Sultan, F.1    Glickstein, M.2
  • 246
    • 53849102608 scopus 로고    scopus 로고
    • Progress in transduction of cerebellar Purkinje cells in vivo using viral vectors
    • H Hirai 2008 Progress in transduction of cerebellar Purkinje cells in vivo using viral vectors Cerebellum 7 3 273 278
    • (2008) Cerebellum , vol.7 , Issue.3 , pp. 273-278
    • Hirai, H.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.